Mature onset diabetes of the young (MODY), refers to any of several heredity forms of diabetes. These forms of diabetes are caused by mutations in a sex independent gene which disrupts the process of insulin production. It can appear in several generations in a family. Mature onset diabetes is also referred to as “monogenic diabetes” according to Wikipedia.org.
Mature onset diabetes affects one to two percent of people who have diabetes. Although all of the normal signs of diabetes are present, this type of diabetes often goes unrecognized. It can be distinguished from type-1 diabetes by establishing the absence of antibodies, and it is not caused by insulin resistance like type-2 diabetes.
Mature onset diabetes, or MODY, can be diagnosed at any age. Patients that are obese tend to get it before the age of 50. Diagnosis requires that the patient meets the following criteria:
- Glucose levels are high
- There are no ketones in the urine
- Antibody tests are negative
- A positive monogenic diabetes test result
Mature Onset Diabetes Types
It is important for a person with mature onset diabetes to understand the different types. This way they can get the proper treatment, understand how it will progress in the future, and other family members will have a better understanding of what they are at risk of inheriting. Six different genes have been identified, they are as follows:
HNF1A – This gene acts as a switch that turns other genes of the body on and off. This gene causes diabetes by lowering the amount of insulin that is produced in the pancreas. It accounts for 70% of the cases of mature onset diabetes.
Glucokinase– This gene plays an important role in the body; it recognizes how high blood sugar levels in the body. When this gene malfunctions, the cells are not signaled to produce insulin, and blood glucose levels run too high.
HNF1B– In this type you produce adamant amounts of insulin as a child, but that amount decreases as you get older. This type is very susceptible to diabetic complications such as eye problems and kidney damage.
HNF4A– This gene is similar to HNF1A, it lowers the amount of insulin that is produced by the pancreas as well.
IPF1– This type is rare; it has only been found in one family in the UK. It produces a fairly mild form of diabetes and there is not much known about it, according to DiabetesExplained.com.
NEUROD1-This gene causes RCAD, or Renal Cysts and Diabetes. When this gene is not working properly it causes familial renal cystic disease and diabetes. It also may cause genital tract malformations and early onset gout.
